Pulmonary arterial hypertension: the burden of disease and impact on quality of life

Abstract

Pulmonary arterial hypertension (PAH) is a debilitating disease that pervades all aspects of a patient's daily life. It is also increasingly acknowledged that the burden of PAH extends to older patients and carers. Until recently, the adverse effect of disease symptoms on the physical, emotional and social factors governing patient health-related quality of life (HRQoL) remained largely unrecognised. With a shift in therapeutic objectives to longer term improvements and HRQoL benefits, clinical trials now frequently include HRQoL measures as study end-points. Most HRQoL instruments used in patients with PAH are generic or non-disease-specific questionnaires and therefore may not accurately capture PAH disease burden. New PAH-specific HRQoL instruments currently undergoing validation include emPHasis-10 and Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland). Using various HRQoL measures, pharmacological therapies have been shown to improve HRQoL in patients with PAH. Patients also derive HRQoL benefits from nonpharmacological strategies, which include the emotional support provided by multidisciplinary care and support groups that is fundamental to patient wellbeing. Looking to the future, validated PAH-specific HRQoL instruments together with dedicated guidelines and procedures are essential to support the translation of HRQoL scores to the clinic, thus enabling a holistic treatment approach to the management of patients with PAH.

FIGURE 1

Effect of pulmonary arterial hypertension (PAH) on SF-36-measured health-related quality of life (HRQoL) measures versus the normal population and other disease conditions [–15]. COPD: chronic obstructive pulmonary disease; SF-36: Medical Outcomes Study 36-item short form.

FIGURE 2

Scoring system for health-related quality of life (HRQoL) scales [33, 40]. SF-36: Medical Outcomes Study 36-item short form; EQ-5D: EuroQol Group 5-Dimension Self-Report Questionnaire; NHP: Nottingham Health Profile; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; MLHFQ: Minnesota Living with Heart Failure Questionnaire; LPH: Living with Pulmonary Hypertension questionnaire; CHQ: Chronic Heart Failure Questionnaire.